Complex Treatment of A Rare Non-Germinomatous Germ Cell Optic-Chiasmal Brain Tumor In An Adolescent Child-A Clinical Case With Literature Review

L. Marinova, V. Vasileva, N. Kahchiev, Katia Sergieva
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Abstract

Non-germ cell tumours (NGCTs) are rare intracranial neoplasms occurring in adolescents and young adults. A multidisciplinary team including a neurosurgeon, oncologist, radiologist, pathohistologist, ophthalmologist, pediatric oncohematologist, neurologist and endocrinologist is required for diagnosis and treatment. We present a rarely diagnosed optic-chiasmal non-germinomatous germ cell tumour (NGGCT) in a 16-year-old boy, accompanied by pituitary insufficiency (diabetes insipidus and central hypothyroidism) and severely impaired vision bilaterally. The immunohistochemistry (IHC) of the tumor biopsy, as well as the level of tumor markers (human chorionic gonadotropin and alpha-fetoprotein) in the serum and cerebrospinal fluid are significant factors for determining the diagnosis-cranial GCT or cranial NGCT. Because the tumor was inoperable, we performed 3 courses of neoadjuvant chemotherapy followed by definitive radiotherapy to a total tumor dose of 55 Gy. In such rare childhood brain tumors, complex treatment achieves long-term local tumor control, requiring long-term monitoring by an ophthalmologist and endocrinologist.
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青少年罕见非肉芽肿性芽胞视神经乳头状脑瘤的复杂治疗--临床病例与文献综述
非胚胎细胞肿瘤(NGCTs)是一种罕见的颅内肿瘤,多发于青少年和年轻成年人。诊断和治疗需要一个多学科团队,包括神经外科医生、肿瘤科医生、放射科医生、病理科医生、眼科医生、儿科肿瘤科医生、神经科医生和内分泌科医生。我们报告的是一名16岁男孩罕见的视桥非绒毛膜生殖细胞瘤(NGGCT),伴有垂体功能不全(糖尿病性尿崩症和中枢性甲状腺功能减退症)和双侧视力严重受损。肿瘤活检的免疫组化(IHC)以及血清和脑脊液中肿瘤标志物(人绒毛膜促性腺激素和甲胎蛋白)的水平是确定诊断(颅内 GCT 或颅内 NGCT)的重要因素。由于肿瘤无法手术,我们进行了 3 个疗程的新辅助化疗,随后进行了肿瘤总剂量为 55 Gy 的确定性放疗。对于这种罕见的儿童脑肿瘤,复杂的治疗可实现长期的局部肿瘤控制,需要眼科医生和内分泌科医生的长期监测。
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